Document Detail

Limbic encephalitis in children and adolescents.
MedLine Citation:
PMID:  20959359     Owner:  NLM     Status:  In-Process    
OBJECTIVE: Limbic encephalitis is rare in people <18 years of age and rarely given a formal diagnosis.
DESIGN: Retrospective study on presentation and outcome of children and adolescents with the clinico-radiological syndrome of limbic encephalitis tested for specific neuronal autoantibodies (Abs) over 3.5 years.
SETTING: Assessment, diagnosis, treatment and follow-up at 12 neuropaediatric and neurological departments in Europe, with Abs determined in Bonn, Germany and Oxford, UK.
PATIENTS: Ten patients <18 years of age who presented with a disorder mainly affecting the limbic areas of <5 years' duration with MRI evidence of mediotemporal encephalitis (hyperintense T2/FLAIR signal, resolving over time).
RESULTS: Median age at disease onset was 14 years (range 3-17). Eight patients had defined Abs: one each with Hu or Ma1/2 Abs, four with high titre glutamic acid decarboxylase (GAD) Abs, two of whom had low voltage-gated potassium channel (VGKC) Abs and two with only low titre VGKC Abs. A tumour was only found in the patient with Hu Abs (a neuroblastoma). After a median follow-up of 15 months with corticosteroid or intravenous immunoglobulin treatment, starting after a median of 4 months, two patients recovered, eight remained impaired and one died.
CONCLUSIONS: Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes.
E Haberlandt; T Bast; A Ebner; H Holthausen; G Kluger; R Kravljanac; J Kröll-Seger; G Kurlemann; C Makowski; K Rostasy; E Tuschen-Hofstätter; G Weber; A Vincent; C G Bien
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Publication Detail:
Type:  Journal Article     Date:  2010-10-19
Journal Detail:
Title:  Archives of disease in childhood     Volume:  96     ISSN:  1468-2044     ISO Abbreviation:  Arch. Dis. Child.     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-01-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372434     Medline TA:  Arch Dis Child     Country:  England    
Other Details:
Languages:  eng     Pagination:  186-91     Citation Subset:  AIM; IM    
Medical University of Innsbruck, Clinical Department of Pediatrics IV, Division of Neuropediatrics and Inherited Metabolic Diseases, Innsbruck, Austria.
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